Oral health care pathways for patients with epidermolysis bullosa: A position statement from the European reference network for rare skin diseases.

Background: Inherited epidermolysis bullosa (EB) comprises a group of genetic disorders characterized by skin fragility and unique oral features. It requires interdisciplinary care from several health professionals, including oral health teams. Modern dentistry encompasses a wide range of therapeutic options performed by specialists from different fields.

Orofacial Anomalies in Kindler Epidermolysis Bullosa.

Importance: Kindler epidermolysis bullosa is a genetic skin-blistering disease associated with recessive inherited pathogenic variants in FERMT1, which encodes kindlin-1. Severe orofacial manifestations of Kindler epidermolysis bullosa, including early oral squamous cell carcinoma, have been reported.

Objective: To determine whether hypoplastic pitted amelogenesis imperfecta is a feature of Kindler epidermolysis bullosa.

Automatic software-based 3D-angular measurement for weight-bearing CT (WBCT) is valid.

Background: The purpose of this study was to compare automatic software-based angular measurement (AM) with validated measurement by hand (MBH) regarding angle values and time spent for Weight-Bearing CT (WBCT) generated datasets.

Methods: Five-hundred WBCT scans from different pathologies were included in the study. 1st - 2nd intermetatarsal angle, talo-1st metatarsal angle dorsoplantar and lateral, hindfoot angle, calcaneal pitch angle were measured and compared between MBH and AM.

Lipin-1 restrains macrophage lipid synthesis to promote inflammation resolution.

Macrophages are critical to maintaining and restoring tissue homeostasis during inflammation. The lipid metabolic state of macrophages influences their function, but a deeper understanding of how lipid metabolism is regulated in pro-resolving macrophage responses is needed. Lipin-1 is a phosphatidic acid phosphatase with a transcriptional coregulatory activity (TC) that regulates lipid metabolism.

Semi-automatic software-based 3D-angular measurement for Weight-Bearing CT (WBCT) in the foot provides different angles than measurement by hand.

Background: The purpose of this study was to compare semi-automatic software-based angular measurement (SAM) with previously validated measurement by hand (MBH) regarding angle values and time spent for the investigator for Weight-Bearing CT (WBCT).

Methods: In this retrospective comparative study, five-hundred bilateral WBCT scans (PedCAT, Curvebeam, Warrington, PA, USA) were included in the study. Five angles (1st - 2nd intermetatarsal angle (IM), talo-metatarsal 1-angle (TMT) dorsoplantar and lateral projection, hindfoot angle, calcaneal pitch angle) were measured with MBH and SAM (Bonelogic Ortho Foot and Ankle, Version 1.

Myeloid-associated lipin-1 transcriptional co-regulatory activity is atheroprotective.

Background And Aims: Atherosclerosis is the most prominent underlying cause of cardiovascular disease (CVD). It is initiated by cholesterol deposition in the arterial intima, which causes macrophage recruitment and proinflammatory responses that promote plaque growth, necrotic core formation, and plaque rupture. Lipin-1 is a phosphatidic acid phosphohydrolase for glycerolipid synthesis.

Quantifying Absolute Neutralization Titers against SARS-CoV-2 by a Standardized Virus Neutralization Assay Allows for Cross-Cohort Comparisons of COVID-19 Sera.

The global coronavirus disease 2019 (COVID-19) pandemic has mobilized efforts to develop vaccines and antibody-based therapeutics, including convalescent-phase plasma therapy, that inhibit viral entry by inducing or transferring neutralizing antibodies (nAbs) against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike glycoprotein (CoV2-S). However, rigorous efficacy testing requires extensive screening with live virus under onerous biosafety level 3 (BSL3) conditions, which limits high-throughput screening of patient and vaccine sera. Myriad BSL2-compatible surrogate virus neutralization assays (VNAs) have been developed to overcome this barrier.

Clinical practice guidelines: Oral health care for children and adults living with epidermolysis bullosa.

Background: Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features.

Aims: To provide (a) a complete review of the oral manifestations in those living with each type of inherited EB, (b) the current best practices for managing oral health care of people living with EB, (c) the current best practices on dental implant-based oral rehabilitation for patients with recessive dystrophic EB (RDEB), and (d) the current best practice for managing local anesthesia, principles of sedation, and general anesthesia for children and adults with EB undergoing dental treatment.

Methods: Systematic literature search, panel discussion including clinical experts and patient representatives from different centers around the world, external review, and guideline piloting.

Macrophage-Associated Lipin-1 Promotes β-Oxidation in Response to Proresolving Stimuli.

Macrophages reprogram their metabolism to promote appropriate responses. Proresolving macrophages primarily use fatty acid oxidation as an energy source. Metabolites generated during the catabolism of fatty acids aid in the resolution of inflammation and tissue repair, but the regulatory mechanisms that control lipid metabolism in macrophages are not fully elucidated.

Interface of Phospholipase Activity, Immune Cell Function, and Atherosclerosis.

Phospholipases are a family of lipid-altering enzymes that can either reduce or increase bioactive lipid levels. Bioactive lipids elicit signaling responses, activate transcription factors, promote G-coupled-protein activity, and modulate membrane fluidity, which mediates cellular function. Phospholipases and the bioactive lipids they produce are important regulators of immune cell activity, dictating both pro-inflammatory and pro-resolving activity.

Quantifying absolute neutralization titers against SARS-CoV-2 by a standardized virus neutralization assay allows for cross-cohort comparisons of COVID-19 sera.

The global COVID-19 pandemic has mobilized efforts to develop vaccines and antibody-based therapeutics, including convalescent plasma therapy, that inhibit viral entry by inducing or transferring neutralizing antibodies (nAbs) against the SARS-CoV-2 spike glycoprotein (CoV2-S). However, rigorous efficacy testing requires extensive screening with live virus under onerous BSL3 conditions which limits high throughput screening of patient and vaccine sera. Myriad BSL-2 compatible surrogate virus neutralization assays (VNAs) have been developed to overcome this barrier.

Alpha-1-antitrypsin and its variant-dependent role in COVID-19 pathogenesis.

Rationale: SARS-CoV-2 entry into host cells is facilitated by endogenous and exogenous proteases that proteolytically activate the spike glycoprotein and antiproteases inhibiting this process. Understanding the key actors in viral entry is crucial for advancing knowledge of virus tropism, pathogenesis, and potential therapeutic targets.

Objectives: We aimed to investigate the role of naïve serum and alpha-1-antitrypsin (AAT) in inhibiting protease-mediated SARS-CoV-2 entry and explore the implications of AAT deficiency on susceptibility to different SARS-CoV-2 variants.

Lipin-1 Contributes to IL-4 Mediated Macrophage Polarization.

Macrophage responses contribute to a diverse array of pathologies ranging from infectious disease to sterile inflammation. Polarization of macrophages determines their cellular function within biological processes. Lipin-1 is a phosphatidic acid phosphatase in which its enzymatic activity contributes to macrophage pro-inflammatory responses.

Knowledge about hand hygiene in the Generation Z: A questionnaire-based survey among dental students, trainee nurses and medical technical assistants in training.

Alcohol-based hand disinfection is the key measure to prevent healthcare-associated infections and nosocomial transmission of pathogens. We conducted a survey among dental students, trainee nurses and medical technical assistants in training from the Generation Z to determine their knowledge regarding hand hygiene and to optimize education. Overall knowledge was worthy of improvement.

Effect of Mandatory 6-Month Waiting Period on Waitlist and Transplant Outcomes in Patients With Hepatocellular Carcinoma.

Background And Aims: Organ Procurement and Transplantation Network/United Network for Organ Sharing (OPTN/UNOS) policy mandates a 6-month waiting period before exception scores are granted to liver transplant candidates with hepatocellular carcinoma (HCC). This study aims to evaluate waitlist and posttransplant outcomes in patients with HCC, before and after implementation of the 6-month waiting rule.

Approach And Results: We examined two groups from the UNOS registry: Group 1 (pre-6-month rule) consisted of patients registered as transplant candidates with HCC from January 1, 2013, to October 7, 2015 (n = 4,814); group 2 (post-6-month rule) consisted of patients registered from October 8, 2015, to June 30, 2018 (n = 3,287).

AAV8-mediated overexpression of mPCSK9 in liver differs between male and female mice.

Background And Aims: The recombinant adeno-associated viral vector serotype 8 expressing the gain-of-function mutation of mouse proprotein convertase subtilisin/kexin type 9 (AAV8- PCSK9) is a new model for the induction of hypercholesterolemia. AAV8 preferentially infects hepatocytes and the incorporated liver-specific promoter should ensure expression of PCSK9 in the liver. Since tissue distribution of AAVs can differ between male and female mice, we investigated the differences in PCSK9 expression and hypercholesterolemia development between male and female mice using the AAV8-PCSK9 model.

Gene Transfer Induced Hypercholesterolemia in Amyloid Mice.

A risk factor for cardiovascular disease (CVD), mutant PCSK9, was expressed in APP/PS1 mice to study the CVD-Alzheimer's disease inter-relationship. Cholesterol levels were elevated by 5-6-fold from 3 to 13 weeks after PCSK9 gene transfer. We tested whether hypercholesterolemia would increase amyloid-β plaques at a relatively early stage of plaque deposition.

Effects of Allocating Livers for Transplantation Based on Model for End-Stage Liver Disease-Sodium Scores on Patient Outcomes.

Background & Aims: The Model for End-stage Liver Disease and Sodium (MELD-Na) score was introduced for liver allocation in January 2016. We evaluated the effects of liver allocation, based on MELD-Na score, on waitlist and post-transplantation outcomes.

Methods: We examined 2 patient groups from the United Network for Organ Sharing registry; the MELD-period group was composed of patients who were registered as transplant candidates from June 18, 2013 through January 10, 2016 (n = 18,850) and the MELD-Na period group was composed of patients who were registered from January 11, 2016 through September 30, 2017 (n = 14,512).

Liver alone or simultaneous liver-kidney transplant? Pretransplant chronic kidney disease and post-transplant outcome - a retrospective study.

The new Organ Procurement and Transplant Network/United Organ Sharing Network (OPTN/UNOS) simultaneous liver-kidney transplant (SLK) policy has been implemented. The aim of this study was to review liver transplant outcomes utilizing the new SLK policy. Liver transplant alone (LTA) and SLK patients between 2009 and 2015 were reviewed.

Variable disease progression after successful stem cell transplantation: prospective follow-up investigations in eight patients with Hurler syndrome.

We report the results of a prospective, standardized follow-up programme of eight children (median age at SCT 1.2 yr) with mucopolysaccharidosis (MPS1H, M. Hurler) transplanted using a fludarabine-based SCT.

Probiotics affect the clinical inflammatory parameters of experimental gingivitis in humans.

Objectives: To determine the effects of a probiotic milk drink consumed over a period of 28 days, regarding the expression of clinical inflammatory parameters of the oral gingiva during various phases of plaque-induced gingivitis.

Methods: Twenty-eight adults with healthy gingiva took part in a prospective and clinical-controlled study. The test group was advised to consume a probiotic milk drink (Yacult) daily during a period of 4 weeks; the control group did not receive any probiotic food or drink.

Shear bond strength of self-etch adhesives to enamel with additional phosphoric acid etching.

This study evaluated the shear bond strength of self-etch adhesives to enamel and the effect of additional phosphoric acid etching. Seventy sound human molars were randomly divided into three test groups and one control group. The enamel surfaces of the control group (n=10) were treated with Syntac Classic (SC).

Identification of a homozygous deletion in the AP3B1 gene causing Hermansky-Pudlak syndrome, type 2.

We report on the molecular etiology of an unusual clinical phenotype associating congenital neutropenia, thrombocytopenia, developmental delay, and hypopigmentation. Using genetic linkage analysis and targeted gene sequencing, we defined a homozygous genomic deletion in AP3B1, the gene encoding the beta chain of the adaptor protein-3 (AP-3) complex. The mutation leads to in-frame skipping of exon 15 and thus perturbs proper assembly of the heterotetrameric AP-3 complex.

Effect of different splinting methods and fixation periods on root development of autotransplanted immature third molars.

Purpose: The aim of the present study was to assess the influence of suture splinting for 1 week or rigid fixation for 4 weeks on final root length, root length increment, and mobility of autotransplanted immature third molars.

Patients And Methods: The sample consisted of 63 patients with a total of 65 transplanted immature third molars. All transplants had reached one-half (n = 29) to three-fourths (n = 36) of their expected root length.